Structural identification of haemoglobin F Kuala Lumpur: 22(B4) ; 136 Ala

Lie‐Injo, Luan Eng; Wiltshire, B.; Lehmann, H.

doi:10.1016/0005-2795(73)90297-3

Cited by 25 publications

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“…Seventeen different abnormal fetal hemoglobins (Hb F,) have been discovered. Seven are G y chain variants [ 10-1 51, seven are A y chain variants [16][17][18][19][20][21][22][23][24] , while three have been incompletely characterized . Most G y Hb F, heterozygotes (except those with Hb F-Port Royal or aZGy2 125 Glu+Ala) have levels of Hb F, which are about twice that of the variant in A y Hb F, heterozygotes (see references quoted, and Table I).…”

Section: Introductionmentioning

confidence: 99%

The synthesis of the ^Gγ and ^Aγ chains of human fetal hemoglobin in erythroid colonies cultured from peripheral blood BFUe's of normal adults and newborn and of subjects with an ^Aγ or a ^Gγ chain abnormal fetal hemoglobin

et al. 1980

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Peripheral blood mononuclear cells from normal adults, normal newborn infants, and from newborn and adult subjects with one of three γ chain variants (GγF‐Malta I, GγF‐Port Royal, AγF‐Hull) were cultured in vitro with erythropoietin. The 35S‐methionine‐labelled hemoglobin from 13‐ to 15‐day‐old BFUe‐derived colonies was studied by chromatography on columns of DEAE‐cellulose and the quantities of Hbs A2, Fx, and F0 determined. The percentages of Gγ and Aγ chains in isolated Hb Fx and Hb F0 were determined using high‐performance liquid chromatography (HPLC) of the tryptic peptides of these proteins. Calculation of these percentages was based on the total activities of the GγT‐15 and AγT‐15 peptides which contain one (35S‐labelled) methionyl residue each and can be separated by the HPLC procedure. The data show an increased synthesis of Hb F in the „adult”︁ colonies and a decreased synthesis in the „newborn”︁ colonies. The Gγ to Aγ ratio of the Hb F from adult colonies varied greatly. The percentages of Gγ and Aγ chains in the Hb F from adult colonies correlated with the percentages in the Hb F isolated from the Hb F of circulating red blood cells. The Gγ to Aγ ratio in the Hb F from newborn colonies was high as in the Hb F from cord blood samples. Gγ and Aγ chain abnormal Hb F variants were readily detectable in colonies from both adults and newborn. The Gγ to Aγ ratio in the Hb F of colonies of adult Hb F‐Malta I and Hb F‐Hull heterozygotes approached 1, but that of adult Hb F‐Port Royal heterozygotes remained about as high as in colonies from newborn heterozygotes. The percent Hb F‐Port Royal in the Hb F of adult colonies was twice that in the Hb F of newborn colonies. These results are discussed in the light of information from recent detailed studies of genomic DNA assuming controlling functions for the segments of DNA that are interspersed between the various structural genes.

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